Autosomal recessive mutations in a number of genes encoding Aminoacyl-tRNA synthetases (ARSs), including HARS2 (Perrault syndrome 2), LARS2 (Perrault syndrome 4), NARS2 (DFNB94), IARS2 (Cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia), PARS2 (Developmental and epileptic encephalopathy 75 including deafness), and KARS (DFNB89) may lead to a variety of syndromic and non-syndromic hearing loss (Konovalova and Tyynismaa, 2013; Oprescu et al., 2017; Wang et al., 2020). The gene discussed is KARS1; the disease is Perrault syndrome.